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ABSTRACT: Purpose
Benzoate and phenylbutyrate are widely used in the treatment of urea cycle disorders, but detailed studies on pharmacokinetics and comparative efficacy on nitrogen excretion are lacking.Methods
We conducted a randomized, three-arm, crossover trial in healthy volunteers to study pharmacokinetics and comparative efficacy of phenylbutyrate (NaPB; 7.15 g•m-2BSA•day-1), benzoate (NaBz; 5.5 g•m-2BSA•day-1), and a combination of two medications (MIX arm; 3.575 g NaPB and 2.75 g NaBz•m-2BSA•day-1) on nitrogen excretion. Stable isotopes were used to study effects on urea production and dietary nitrogen disposal.Results
The conjugation efficacy for both phenylbutyrate and benzoate was 65%; conjugation w
SUBMITTER: Nagamani SCS
PROVIDER: S-EPMC5924481 | biostudies-literature | 2018 Jul
REPOSITORIES: biostudies-literature